Clinicopathological consultation: Review of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) of head and neck

Antonino Carbone, Alberto Passannante, Annunziata Gloghini, Kenneth O. Devaney, Alessandra Rinaldo, Alfio Ferlito

Research output: Contribution to journalArticle

Abstract

The entity known as sinus histiocytosis with massive lymphadenopathy (SHML), or Rosai-Dorfman disease (RD disease), is an uncommon benign proliferation of hematopoietic and fibrous tissue that often presents in the head and neck region. Its initial manifestations most often include a roughly symmetric, painless, bilateral cervical adenopathy, although extranodal disease may develop in a minority of patients. The key histologic feature of SHML is the presence of various numbers of large, pale histiocytic cells that contain within their cellular borders apparently engulfed lymphocytes ('emperipolesis'); these distinctive large, pale cells - RD cells - are S-100 protein-positive by immunostaining and so differ from ordinary histiocytes. Despite its sometimes impressive clinical presentation, SHML is a benign and self-limited disease, whose treatment is aimed largely at controlling its local manifestations (most often by surgical therapy). The microscopic differential diagnosis, particularly in extranodal disease, is at times challenging and can include Langerhans' cell histiocytosis, Hodgkin's disease, non-Hodgkin's lymphoma, metastatic carcinoma, and metastatic malignant melanoma.

Original languageEnglish
Pages (from-to)1095-1104
Number of pages10
JournalAnnals of Otology, Rhinology and Laryngology
Volume108
Issue number11 I
Publication statusPublished - 1999

ASJC Scopus subject areas

  • Otorhinolaryngology

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